The Neurological Center: Metabolic/Degenerative Disorders is a multidisciplinary center bringing together clinicians and scientists representing a broad range of disciplines: biochemistry, organic chemistry, electron microscopy, histochemistry, mathematics, neurophysiology, neuropsychology, physiology of cerebral blood flow; to study neurologic disease processes and basic phenomena underlying these diseases. This program project is concerned with the inherited metabolic and developmental disorders, including sphingolipidoses, adrenal leukodystrophy, and related disease. Collaborative studies of ultrastructure and chemical changes being conducted are aimed at understanding the molecular basis of these diseases. Complementing these, are studies of appropriate experimental disease models and relevant fundamental aspects of appropriate experimental disease models and relevant fundamental aspects of the neurochemistry of involved cellular and subcellular structures. Neuropsychological studies are directed primarily at understanding the nature of the memory and cogntitive defects that are the principal manifestations of the dementias. Changes in regional blood flow that may serve as a measure of neuronal and metabolic dysfunction underlying psychological defects are being studied.